Zebrafish Corneal Dystrophy Models

Corneal dystrophies are a genetically heterogeneous group of diseases, a collection of diseases classified by affected corneal layers, and the mechanisms by which they occur are poorly understood. They are non-inflammatory, hereditary and cause opacity. Despite extensive embryological and genetic analysis of the zebrafish retina, little is known about the zebrafish cornea and the genetic mechanisms underlying its differentiation. Understanding the mechanisms by which cornea differentiates and maintains its structure and function has great fundamental scientific and medical implications.

Examples of corneal dysplasia in zebrafish have been reported, but in general the phenotypes described so far are generally more severe than the human disease state. For example, mutations in genes encoding proteins related to apical-basal cell polarity and the integrity of the apical junction complex lead to hypoplasia of the corneal epithelium and/or stroma. Zebrafish have been used to study the function of genes whose mutations cause corneal dystrophy because of their genetic accessibility. Some genes that cause corneal dystrophy in humans, such as pip5k3, col17a1, and keratocan, are also expressed in zebrafish corneas. Likewise, deletion of lama1, a gene encoding an important basement membrane protein, causes focal corneal dysplasia in zebrafish.

Fig.1 The adult zebrafish cornea at 6 mpf contains all five major layers found in the human cornea.

Our Zebrafish Corneal Dystrophy Models

Creative Biogene provides a series of transgenic zebrafish corneal dystrophy models, such as the insertion of a BIGH3 gene containing a human dominant mutation into the zebrafish genome through transgenic to produce corneal abnormalities associated with human dystrophy. This transgenic zebrafish line can be used to screen for chemicals that alleviate corneal defects and provide a basis for analyzing the underlying genetic mechanisms of corneal development and studying the causes of corneal diseases. In addition, we provide detailed analysis of corneal development and differentiation in zebrafish using light and electron microscopy and immunohistochemical techniques to assist you in understanding corneal differentiation and the mechanisms that maintain its structure and function.

Advantages

• Genetic screening of mutants for embryonic development
• Efficiently perform positional cloning
• Ease of loss-of-function genetic analysis
• Real-time observation of disease progression

Creative Biogene aims to provide a valuable tool for studying the etiology of corneal dystrophy and developing new drugs as an affordable high-throughput system. If you would like to learn more about zebrafish models, please feel free to contact us.

References

1. Zhao XC, et al. The zebrafish cornea: structure and development. Invest Ophthalmol Vis Sci. 2006, 47(10):4341-4348.
2. Gestri G, Link BA, Neuhauss SC. The visual system of zebrafish and its use to model human ocular diseases. Dev Neurobiol. 2012, 72(3):302-27.
3. Richardson R, et al. The zebrafish eye-a paradigm for investigating human ocular genetics. Eye (Lond). 2017, 31(1):68-86.

For research use only. Not intended for any clinical use.